Endothelin Receptor Antagonist

2020 ◽  
Author(s):  
Keyword(s):  
Receptor Antagonist ◽  
Endothelin Receptor Antagonist ◽  
Endothelin Receptor

scholarly journals Adolescents and adults with Fontan circulation: insights from the PREpArE-Fontan registry

2021 ◽  
pp. 1-9
Author(s):  
Lars Søndergaard ◽  
Jamil Aboulhosn ◽  
Yves d’Udekem ◽  
Céline Faure ◽  
Wayne J Franklin ◽  
...  
Keyword(s):  
Creatinine Clearance ◽  
Receptor Antagonist ◽  
Functional Class ◽  
Fontan Circulation ◽  
Endothelin Receptor Antagonist ◽  
Endothelin Receptor ◽  
Converting Enzyme Inhibitors ◽  
Nyha Functional Class ◽  
Lateral Tunnel ◽  
Adolescents And Adults

Abstract The Patient Registry for Adolescents and Adults with Stable Fontan Circulation aims to describe a contemporary cohort of Fontan patients who could be eligible for a clinical trial investigating macitentan, an endothelin receptor antagonist. This international, non-interventional, multicentre, cross-sectional, observational registry enrolled patients with “stable” Fontan circulation ≥10 years following extra-cardiac conduit or lateral tunnel procedure. Main exclusion criteria were NYHA functional class IV, reoperation of Fontan circulation, or signs of disease worsening. Patient characteristics at enrolment are described; available data were collected during a single registration visit. Of the 266 screened patients, 254 were included in this analysis. At enrolment, median (interquartile range) age was 24 (20;30) years, 37%/63% of patients were from the USA/Europe, 54% were male, 54%/47% had undergone extra-cardiac conduit/lateral tunnel procedures, and 95% were in NYHA functional class I or II. History of arrhythmia was more common in older patients and patients with lateral tunnel; overall prevalence was 19%. Most laboratory values were within the normal range but mean creatinine clearance was abnormally low (87.7 ml/min). Angiotensin-converting enzyme inhibitors were used by 48% of patients and their use was associated with creatinine clearance <90 ml/min (p = 0.007), as was Fontan completion at an older age (p = 0.007). 53.4% of patients had clinical characteristics that could potentially meet an endothelin receptor antagonist trial’s eligibility criteria. The PREpArE-Fontan registry describes a cohort of patients who could potentially participate in an endothelin receptor antagonist trial and identified early subtle signs of Fontan failure, even in “stable” patients.

scholarly journals Multidimensional fatigue in pulmonary hypertension: prevalence, severity and predictors

2018 ◽  
Vol 4 (1) ◽  
pp. 00079-2017 ◽  
Author(s):  
Todd M. Tartavoulle ◽  
Aryn C. Karpinski ◽  
Andrew Aubin ◽  
Benzi M. Kluger ◽  
Oliver Distler ◽  
...  
Keyword(s):  
Body Mass Index ◽  
Pulmonary Hypertension ◽  
Receptor Antagonist ◽  
Body Mass ◽  
Phosphodiesterase Inhibitor ◽  
Endothelin Receptor Antagonist ◽  
Endothelin Receptor ◽  
Physical Fatigue ◽  
General Fatigue ◽  
Reduced Activity

Pulmonary hypertension is a potentially fatal disease. Despite pharmacological advances in pulmonary hypertension, fatigue remains common in patients with pulmonary hypertension.A convenience sample of 120 participants at an international patient conference completed the Multidimensional Fatigue Inventory (MFI)-20 scale. Data on New York Heart Association Functional Class, body mass index, oxygen use and medication type/use were also collected.There was a high prevalence of “severe” to “very severe” fatigue for each dimension: General Fatigue (60%), Physical Fatigue (55.8%), Reduced Activity (41.7%), Reduced Motivation (32.5%) and Mental Fatigue (27.5%). The mean±sd overall MFI-20 score was 58±5.1. Dimensions with the highest averaged levels were General Fatigue (13.40±3.61), Physical Fatigue (13.23±3.67) and Reduced Activity (11.33±4.16). Body mass index correlated with higher fatigue scores. Phosphodiesterase inhibitor plus endothelin receptor antagonist combination negatively predicted General Fatigue, Physical Fatigue, Reduced Motivation and Reduced Activity. Triple therapy was a significant predictor of General Fatigue, Physical Fatigue and Reduced Activity. There were no significant predictors of Mental Fatigue.Multidimensional fatigue is common and severe in patients with pulmonary hypertension. Phosphodiesterase inhibitor plus endothelin receptor antagonist combination resulted in lower scores in most fatigue dimensions. Comprehensive assessment of fatigue should be considered in the clinical care of patients with pulmonary hypertension and clinical research to develop formal interventions that target this disabling symptom.

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